ABSTRACT
Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively.
A anemia de Fanconi é uma síndrome hereditária autossômica recessiva rara, caracterizada por deficiência da medula óssea e anomalias congênitas e hematológicas. A literatura sobre o manejo anestésico dos pacientes é limitada. O manejo de uma displasia do desenvolvimento do quadril foi descrito em um paciente com anemia de Fanconi. Por causa da natureza heterogênea, um paciente com anemia de Fanconi deve ser submetido à avaliação pré-operatória para diagnosticar as características clínicas. Em conclusão, o bloqueio caudal foi a nossa escolha para esse paciente com anemia de Fanconi, sem trombocitopenia, para evitar o N2O, reduzir a quantidade de anestésico, a microcefalia existente, o hipotireoidismo e o aumento das enzimas hepáticas, proporcionar analgesia pós-operatória e reduzir a quantidade de analgésico usada no pós-operatório.
La anemia de Fanconi es un síndrome hereditario autosómico recesivo raro, caracterizado por deficiencia de la médula ósea y por anomalías congénitas y hematológicas. La literatura sobre el manejo anestésico de esos pacientes es limitada. El manejo de una displasia del desarrollo de la cadera fue descrito en un paciente con anemia de Fanconi. Debido a la naturaleza heterogénea, un paciente con anemia de Fanconi debe ser sometido a la evaluación preoperatoria para diagnosticar las características clínicas. En conclusión, el bloqueo caudal fue nuestra elección para ese paciente con anemia de Fanconi sin trombocitopenia para evitar el N2O, reducir la cantidad de anestésico, microcefalia existente, hipotiroidismo y aumento de las enzimas hepáticas, proporcionar analgesia postoperatoria y reducir la cantidad de analgésico usado en el postoperatorio.
Subject(s)
Child, Preschool , Female , Humans , Anesthesia, Caudal/methods , Fanconi Anemia/surgery , Hip Dislocation, Congenital/surgery , Analgesics/administration & dosage , Analgesics/therapeutic use , Fanconi Anemia/physiopathology , Hip Dislocation, Congenital/etiology , Pain, Postoperative/drug therapyABSTRACT
Objective: To study the risk factors for hemangioma-related complications, treatment indications and analyze the outcome of patients with infantile hemangioma. Design: Retrospective. Setting: University hospital. Patients: Fifty-five patients (1-69 months; median: 12 months) with infantile hemangioma with mean follow-up 19 months. The eligibility was based on the criteria of the International Society for the Study of Vascular Anomalies (ISSVA). Intervention: The surgical treatment included total excision whereas medical treatment was carried out by interferon and /or corticosteroids. Main outcome measures: Data was collected including sex, age, prematurity, age at onset, number, anatomic location and size of hemangioma, age at treatment, cause of treatment decision, family history, presence of extra malformations, involvement of internal organs, presence of life altering or life threatening complications, response to treatment, dose and duration of medications, complications associated with treatment, follow-up period, and final outcome. Results: Thirty-four (62%) patients were followed-up without treatment, whereas 21 others underwent treatment including steroids, interferon, and surgery. The size of hemangioma was a major factor that predicted hemangioma-related complications (P=0.002). Patients with hemangioma related complications had bigger lesions (size ≥40cm2 or the longest size on a single plane ≥5 cm). Nineteen patients (34%) had complications, but only 8 (14.5%) out of them had life or function-threatening complications. Conclusion: Although dosing and treatment protocol is still debatable, steroids and interferon are good options for hemangioma treatment. The management strategy should be individualized for each case.